Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression

Background: Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that usually presents as rapidly progressive glomerulonephritis, often with pulmonary hemorrhage (the Goodpasture syndr ome). It is reported that patients with severe renal failure do not general ly recover renal function. Objective: To examine the long-term outcome of severe anti-GBM antibody dis ease. Design: Retrospective review of patients treated for confirmed anti-GBM ant ibody disease over 25 years. Setting: A tertiary referral center in the United Kingdom. Patients: 71 treated patients with anti-GBM antibody disease. Intervention: All patients received plasma exchange, prednisolone, and cycl ophosphamide, Measurements: Patient and renal survival, renal histology, and antibody lev els. Results: Patients who presented with a creatinine concentration less than 5 00 mu mol/L (5.7 mg/dL) (n = 19) had 100% patient survival and 95% renal su rvival at 1 year and 84% patient survival and 74% renal survival at last fo llow-up. In patients who presented with a creatinine concentration of 500 m u mol/L or more (greater than or equal to5.7 mg/dL) (n = 13) but did not re quire immediate dialysis, patient and renal survival were 83% and 82% at 1 year and 62% and 69% at last follow-up. In patients who presented with dial ysis-dependent renal failure (n = 39), patient and renal survival were 65% and 8% at 1 year and 36% and 5% at last follow-up. All patients who require d immediate dialysis and had 100% crescents on renal biopsy remained dialys is dependent. Conclusions: Patients with the Goodpasture syndrome and severe renal failur e should be considered for urgent immunosuppression therapy, including plas ma exchange, to maximize the chance of renal recovery. Patients needing imm ediate dialysis are less likely to recover.